Two cases of intramedullary paracoccidioidomycosis are reported. Paracoccidioidomycosis is a systemic disease that involves the buccopharyngeal mucosa, lungs lymph nodes and viscera and infrequently the central nervous system. Localization in the spinal cord is rare. Case 1: a 55-year old male admitted with crural pararesis, tactile/painful hypesthesia and sphincter disturbances of 15 days duration. Cutaneous-pulmonary blastomycosis was diagnosed 17 years ago. Myelotomography showed a blockade of T3-T4 (intramedullary lesion). The lesion surgically removed was a Paracoccidioides brasiliensis granuloma. Treatment with sulfadiazine was started after the surgery. Follow-up of 15 month showed an improvement of the clinical signs. Case 2: a 57-year old male was admitted elsewhere 6 months ago and, with a radiologic diagnosis of pulmonary paracoccidioidomycosis, was treated with amphotericin B. He progressively developer paresthesia and tactile/pain anaesthesia on the left side, sphincter disturbances and tetraparesis with bilateral extensor plantar response and clonus of the feet. Myelotomography showed a blockade of C4-C6 (intramedullary lesion). The lesion was not found during surgical exploration and the patient deteriorated and died. Post-mortem examination revealed an intramedullary tumor above the site of the mielotomy (Paracoccidioides brasiliensis granuloma). The preoperative diagnosis of intramedullary paracoccidioidomycotic granulomas is difficult because the clinical and radiologic manifestations are uncharacteristic. Clinical suspicion was possible in our cases based on the history of previous systemic disease. Contrary to intracranial localizations, paracoccidioidomycotic granulomas causing progressive spinal cord compression may require early surgery because response to clinical treatment is slow and the reversibility of neurological deficits depends on the promptness of the decompression.